So How do you cure MSUD?
1. DIETARY RESTRICTION OF THE BCAAs ( leucine,isoleucine and valine). This restriction must start from young to prevent potential brain damage. Babies with MSUD must eat a BCAA-free formula. As the patient grows up, he/she must carefully watch their diet and avoid HIGH PROTEIN foods such as meat or eggs. vegetarians pretty much!! :(
2.However, if the 3 AAs' levels are still too high, patients can undergo dialysis to remove the excess BCAAs in their bodies or inject solutions into the patient's bloodstream to stimulate the usage of the excess BCAAs for muscle protein synthesis.
3. Another potential cure is GENE THERAPY. This would involve incorporating good copies of the gene into the patient, allowing the patient's cells to generate a functional BCKD protein complex and break down the excess AAs. However, it is not exactly safe because it might induce mutations elsewhere in the patient's body and cause other problems.
1. DIETARY RESTRICTION OF THE BCAAs ( leucine,isoleucine and valine). This restriction must start from young to prevent potential brain damage. Babies with MSUD must eat a BCAA-free formula. As the patient grows up, he/she must carefully watch their diet and avoid HIGH PROTEIN foods such as meat or eggs. vegetarians pretty much!! :(
2.However, if the 3 AAs' levels are still too high, patients can undergo dialysis to remove the excess BCAAs in their bodies or inject solutions into the patient's bloodstream to stimulate the usage of the excess BCAAs for muscle protein synthesis.
3. Another potential cure is GENE THERAPY. This would involve incorporating good copies of the gene into the patient, allowing the patient's cells to generate a functional BCKD protein complex and break down the excess AAs. However, it is not exactly safe because it might induce mutations elsewhere in the patient's body and cause other problems.
References
Genetic Science Learning Center (1969, December 31) Maple Syrup Urine Disease (MSUD). Learn.Genetics. Retrieved June 18, 2012, from http://learn.genetics.utah.edu/content/disorders/whataregd/msud/
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